Grand Rounds Niloofar Piri, MD Jan 17th 2014

 CC: Blind spots and blurry vision OU for more than 2 years (OS more severely affected)  HPI: A 74-y Caucasian gentleman presented with gradually increasing visual field defect and night blindness OU for more than two years. He reports episodes of exacerbation which never returned to normal  He claims a history of blunt trauma OS in 1960s after which the vision OS has been low, he has noticed deterioration of VA OS as well for the past two years.

 PMH: CAD -  Coronary angioplasty x2 (1999, 2006)  DH: Verapamil, ASA 83 mg/d, Crestor ( Statin)

Ocular examination  BCVA OD: 20/40 ( x90) OS: CF 1m  Anterior segment OD : NS 1+, CC1+ OS: pc-IOL  IOP 14 mmHg 18 mmHg No cells in the anterior chamber or anterior vitreous

Color Photo Peripapillary atrophy OS>OD and pigment clumping OS>OD

OCT OD June 2013 Epiretinal membrane, Loss of outer hyper-reflective bands outside the fovea, central outer layers preserved

OS OCT Loss of outer retinal layers reflected as loss of outer hyper-reflective bands, central thinning

Autofluorescence: peripapillary hypo-autofluorescence OS>OD, multiple concentric rings of hypo and hyper-autofluorescence extending from the center to the arcades

Multifocal ERG

First order response density demonstrates significantly reduced cone functions OU (OS>OD)

Full field ERG demonstrates almost completely abolished scotopic and photropic and flicker responses OS, as well as diminished amplitudes OD

 Assessment: A 74-y old Caucasian gentleman with a history of progressive visual field loss and nyctalopia with some episodes of exacerbations for the past 2 years OS>>OD

 Impression: (AAOR)  AZOOR (Acute Zonal Occult Outer Retinopathy) (Acute Annular Outer Retinopathy (AAOR) variant)  AIR (Autoimmune Retinopathy)(CAR, MAR, npAIR)

Monson DM, Smith JR. Acute Zonal Occult Outer Retinopathy (AZOOR) : Major review. Surv of Ophthalmology 2011  In 1992, Gass reported a ‘syndrome’ characterized by the sudden onset of photopsia and acute scotomas related to loss of sectors of outer retinal function

retinal pigment epithelial atrophy pigment clumping  Early in the course of the disease, funduscopic appearance was often normal; however, most patients developed zones of retinal pigment epithelial atrophy or pigment clumping  Changes in the electroretinogram (ERG) and persistent visual field defects were usually observed. Although the retinal dysfunction in AZOOR is focal clinically, the ERG changes suggested a global deficit.

 Acute onset of decreased vision in a zone of the visual field, usually accompanied by photopsia  Visual field defect and the photopsia worsened in bright light

 76% in women, mean age 37 (17-79)  Starts unilaterally in 62% but progresses to bilateral disease from several weeks to 228 months

 In the group of 51 patients followed by Gass et al,20 stabilization of visual field loss occurred within 6 months for 77% of patients.

 In 1995 Gass and Stern described acute annular outer retinopathy (AAOR) as a variant of AZOOR

absence of photopsia  The clinical findings of AAOR were considered to be the same as AZOOR, except for the absence of photopsia and the development of an evanescent annular opacity in the retina.

 No known treatment is proved to be effective.